8910/3 Embryonal rhabdomyosarcoma, NOS

Related terms

Definitions

Vulva

ICD-O-3 topography code:

C51

ICD10:

C51

Rhabdomyosarcomas are the most frequent malignant soft tissue tumours in children and adolescents, with embryonal rhabdomyosarcoma as the most common subtype. Typical sites of involvement are the head and neck region and the genitourinary system. Phenotypically and biologically, embryonal rhabdomyosarcomas recapitulate features of embryonal skeletal muscle. Genetically, most cases show loss of chromosomal region 11p15 1

Vulvar embryonal rhabdomyosarcomas are found almost exclusively in girls <10 years of age. The tumours typically arise from the labial or perineal area and form solid masses. Patients present with bleeding and ulceration. The prognosis depends on the clinical stage and the histological pattern. An alveolar histology is associated with a poor prognosis, whereas the survival rate for classic embryonal rhabdomyosarcoma is >90% 3

Rhabdomyosarcomas are the most frequent malignant soft tissue tumours in children and adolescents, with embryonal rhabdomyosarcoma as the most common subtype. Typical sites of involvement are the head and neck region and the genitourinary system. Phenotypically and biologically, embryonal rhabdomyosarcomas recapitulate features of embryonal skeletal muscle. Genetically, most cases show loss of chromosomal region 11p15 6

Rhabdomyosarcomas are the most frequent malignant soft tissue tumours in children and adolescents, with embryonal rhabdomyosarcoma as the most common subtype. Typical sites of involvement are the head and neck region and the genitourinary system. Phenotypically and biologically, embryonal rhabdomyosarcomas recapitulate features of embryonal skeletal muscle. Genetically, most cases show loss of chromosomal region 11p15 8

Rhabdomyosarcomas are the most frequent malignant soft tissue tumours in children and adolescents, with embryonal rhabdomyosarcoma as the most common subtype. Typical sites of involvement are the head and neck region and the genitourinary system. Phenotypically and biologically, embryonal rhabdomyosarcomas recapitulate features of embryonal skeletal muscle. Genetically, most cases show loss of chromosomal region 11p15 10

Cervical embryonal rhabdomyosarcoma (sarcoma botryoides) typically grows in a polypoid fashion. The polypoid masses may be pedunculated or sessile. They range in size from 2-10 cm. Small haemorrhagig areas as well as islands of mature neoplastic cartilage may be observed.
Sarcoma botryoides is rare. It usually arises in children and young adults (mean age 18 years). The prognosis is better than for other types of cervical sarcoma11